Adrenogenital Syndrome
1 medicine
Adrenogenital syndrome is an inherited adrenal disorder, most commonly congenital adrenal hyperplasia, that causes abnormal hormone production and is managed with lifelong steroid and hormone replacement.
Key facts
- Adrenogenital syndrome is an inherited condition in which the adrenal glands can't make cortisol efficiently, causing a build-up of androgens (male sex hormones).
- The most common form is congenital adrenal hyperplasia (CAH); it can cause ambiguous genitalia at birth in girls, early puberty, and rapid early growth that paradoxically leads to shorter adult height.
- A faulty enzyme blocks normal adrenal hormone production, and in the salt-wasting form, sodium and blood pressure can drop dangerously.
- Corticosteroids suppress excess androgen production, and fludrocortisone replaces aldosterone in the salt-wasting form; treatment is generally lifelong with regular monitoring.
What goes wrong in the adrenal glands
The adrenal glands normally make cortisol and aldosterone. In adrenogenital syndrome, a faulty enzyme blocks this process. The body responds by overdriving the adrenal glands, which then overproduce androgens instead. In girls this can cause ambiguous genitalia at birth; in boys it may go unnoticed initially. Both sexes can experience early puberty, rapid early growth that paradoxically results in shorter adult stature, and, in the salt-wasting form, dangerous drops in sodium and blood pressure. The most common form, congenital adrenal hyperplasia (CAH), affects children and adults across all populations.
Managing the condition over time
Treatment centres on replacing the hormones the adrenal glands can't make adequately. Corticosteroids suppress the excess androgen production, while fludrocortisone replaces aldosterone in those with the salt-wasting form, helping the kidneys retain sodium and maintain blood pressure. Both medicines are generally continued lifelong, and doses are adjusted during illness, surgery, or periods of physical stress. Regular monitoring of growth, blood pressure, and hormone levels is essential, particularly in children.
When to seek urgent care
Anyone with adrenogenital syndrome who develops sudden vomiting, extreme fatigue, or collapse needs urgent medical attention, as these can signal an adrenal crisis.
This page is educational and does not replace advice from a doctor or pharmacist who knows your health history.